Long-Term Aortic Valve Function in Patients With or Without Surgical Treatment for Discrete Subaortic Stenosis.

The purpose of this study was to evaluate the long-term function of the aortic valve in patients with discrete subaortic stenosis (DSS), and its prognostic implications. 34 patients with the diagnosis of isolated DSS were treated at our pediatric heart center between 1992 and 2019. Demographic, echocardiographic and surgical data were analyzed. 26 patients had surgery to remove the subaortic membrane. The primary endpoint of this study was a change in the aortic valve function, secondary outcomes included functional class, aortic valve replacement, and reoperation due to recurrence of DSS. The mean post-operative follow-up time was 7.07 years (1-22.5). At the time of the most recent follow up, in the surgical group 11 patients (46%) had echocardiographic evidence of deterioration in their aortic valve insufficiency (AI) (p < 0.002). The deterioration was associated with a pre-operative left ventricular outflow tract (LVOT) gradient greater than 49 mmHg (p < 0.022). 8 patients with DSS were followed for a mean of 12.9 years (6.8-21.3), without meeting the criteria for surgical intervention, none showed any change in the aortic valve function during follow-up time. DSS resection may not prevent worsening of aortic valve insufficiency over time. Patients with stable AI and low LVOT gradient may not require surgery. Given these findings, it may be appropriate to carefully follow patients with new onset AI and low LVOT gradient, delaying surgical intervention.

Surgical outcome in aortopulmonary window beyond the neonatal period.

OBJECTIVE: Aortopulmonary window (APW) is a rare congenital cardiac defect accounting for 0.1% to 0.2% of all congenital cardiac defects. We here present the current midterm outcome of surgical repair of APW in patients more than 3 months of age. METHODS: The retrospective study was conducted to identify all the patients more than 3 months of age at presentation who underwent surgical repair of APW between June 2010 and August 2018 at our tertiary care institute and their outcome was analyzed. RESULTS: We found 14 patients of APW operated at the age of more than 3 months over a period of 8 years. Mean age of the cohort was 2.29 ± 2.96 years ranging from 3 months to 10 years with 57.14% being males. There were 11 (78.57%) patients with isolated APW and 3 (21.43%) had associated cardiac defects including tetralogy of Fallot (n = 1), ventricular septal defect (n = 1), subaortic membrane causing subaortic stenosis (n = 1), and one had extracardiac malformations. Two patients had type I, nine had type II, and three had type III APW as per Jacobs' classification. The mean size of the defect was 14.14 ± 4.33 mm. Mean duration of mechanical ventilation was 26.91 ± 16.65 hours (range, 12.25-67 hours). There was one in-hospital mortality and no late mortality over a mean follow-up of 3.06 ± 2.19 years. None of the patients required any kind of reintervention. CONCLUSION: Good results can be obtained even on late presentation with adequate perioperative care of the patients with the reversible pulmonary hypertensive disease.

Correction of left ventricular outflow tract obstruction caused by anomalous papillary muscle and subaortic membrane

Abstract This paper presents a case study of a 30-year-old male patient with dyspnea on exertion had echocardiographic diagnosis of aortic subvalvar stenosis. Discrete mitral regurgitation and aortic valve dysplasia with mild to moderate insufficiency and hypertrophic cardiomyopathy were also noted. During surgery, a rare condition was identified: presence of papillary muscle anomaly associated with the subaortic membrane as a cause of obstruction of the left ventricular outflow tract. With the resection of these structures and a mitral valve annuloplasty, the patient evolved with a significant improvement of clinical condition and heart failure, with no residual mitral insufficiency.

Membranous subaortic stenosis coexisting with hypertrophic cardiomyopathy: A case report.

Subaortic stenosis (SAS) is a congenital heart disease, and its association with hypertrophic cardiomyopathy is very rare and clinically underappreciated. We report here a case of a 45-year-old female who was admitted to our hospital with chest tightness and shortness of breath. Both transthoracic and transesophageal echocardiography revealed asymmetric left ventricular hypertrophy and a membrane-like echo below the level of the aortic valve. This patient was diagnosed with membranous SAS with hypertrophic cardiomyopathy. Screening of her immediate family members revealed that her son also had hypertrophic cardiomyopathy. The patient chose conservative treatments and has been closely followed-up after discharge.

56-year-old lady with transaortic gradient.

CLINICAL INTRODUCTION: A 56-year-old lady with a background of hypertension was admitted to our institution with acute pulmonary oedema. She reported gradual and increasingly severe dyspnoea on exertion over the preceding 12 months and, prior to presentation, her exercise tolerance was restricted to one flight of stairs. On transthoracic echocardiography during the index admission, left ventricular size and systolic function were normal, and peak and mean transaortic gradients were 67 mm Hg and 33 mm Hg, respectively, with a peak velocity of 3.9 m/s. No aortic incompetence or other significant valvular abnormality was noted. A transoesophageal echocardiogram was performed. Figure 1 depicts the mid-oesophageal parasternal long-axis view. What is the explanation behind the significant transaortic gradient?heartjnl;104/12/1036/F1F1F1Figure 1Transoesophageal echocardiogram, mid-oesophageal long-axis view at 135 degrees. QUESTION: What is the explanation behind the significant transaortic gradient?Ventricular septal defect Supravalvular aortic stenosisAortic valvular stenosisSubaortic membraneHypertrophic obstructive cardiomyopathy.

Causes of an increased pressure gradient through the left ventricular outflow tract: a West Coast experience.

BACKGROUND: Left ventricular outflow tract obstruction (LVOTO) occurs from not only obstructive hypertrophic cardiomyopathy but also other conditions such as sigmoid septum or post mitral valve repair. However, the changes of the LVOT pressure gradient (LVOT PG) in LVOTO with various conditions remain unclear. METHODS: The clinical characteristics and echocardiographic parameters of 73 patients with LVOT PG ≥50 mmHg at rest on Doppler ultrasound were retrospectively investigated. RESULTS: In these patients (age 69 ± 15 years, 38% male), high prevalences of hypertension (66%) and anemia (43%) were observed. The most frequent clinical disease causing LVOTO was hypertrophic obstructive cardiomyopathy (HOCM) (74%). There were other conditions, including hypertensive left ventricular hypertrophy (9%), post-open heart surgery (7%), sigmoid septum (4%), hyperkinetic LV (3%), takotsubo cardiomyopathy (1.5%), and discrete subaortic membrane (1.5%). Significant improvement or reduction of LVOTO was observed in 93% of cases at follow-up (mean 44 months) echocardiography compared with the initial one with the use of medications and transcatheter procedures. CONCLUSIONS: The causes of LVOTO are diverse. However, the occurrence of LVOTO might depend on the coexistence of primary morphological LV characteristics and hemodynamic LV status. Specific factors causing LVOTO need to be investigated, and efforts for improvement of each individual status by the appropriate approach are required.

Correction of Left Ventricular Outflow Tract Obstruction Caused by Anomalous Papillary Muscle and Subaortic Membrane.

This paper presents a case study of a 30-year-old male patient with dyspnea on exertion had echocardiographic diagnosis of aortic subvalvar stenosis. Discrete mitral regurgitation and aortic valve dysplasia with mild to moderate insufficiency and hypertrophic cardiomyopathy were also noted. During surgery, a rare condition was identified: presence of papillary muscle anomaly associated with the subaortic membrane as a cause of obstruction of the left ventricular outflow tract. With the resection of these structures and a mitral valve annuloplasty, the patient evolved with a significant improvement of clinical condition and heart failure, with no residual mitral insufficiency.

Acquired discrete subaortic stenosis in solid organ transplant recipients.

DSS is a rare acquired abnormality of the LV outflow tract in children and adults. DSS can be seen with or without associated congenital heart disease, but the mechanism of its emergence has been poorly understood. Here, we report two cases of DSS that developed after solid organ transplant in childhood. One patient underwent liver and kidney transplant at three yr of age for hereditary polycystic disease, and the other had liver transplant at three months of age due to uncontrollable high-output failure secondary to a large hepatic hemangioma. Both patients developed mild dynamic LV outflow tract obstruction and systemic hypertension soon after transplant, both of which resolved by the time of discharge. Transient LV hypertrophy was observed in both cases. Corticosteroids, cyclosporine A, and tacrolimus were used as initial immunosuppressants. Both patients developed DSS long after the regression of the initial dynamic LV outflow tract obstruction. Herein, we report DSS as an atypical late cardiovascular complication after solid organ transplant and postulate that transient post-transplant hyperdynamic LV outflow tract obstruction and chronic immunosuppressive treatment may lead to subendocardial remodeling resulting in a fibromuscular membranous ridge in susceptible subjects.

[Discrete type subaortic stenosis disclosed by hemolytic anemia after aortic and mitral valve replacement].

We report a case of discrete type subaortic stenosis disclosed by hemolytic anemia 7 years after aortic and mitral prosthetic valve replacement. A 53-year-old female complained of general fatigue, dyspnea, macrohematuria and hemolysis. She had undergone aortic valve replacement for non-coronary cusp perforation 15 years before, and mitral valve replacement and tricuspid annuloplasty 7 years before. Echocardiography showed mitral prosthetic valve regurgitation (III/IV degree) and symptomatic hemolysis might be caused by accelerated blood flow through the prosthetic valve. A mild aortic stenosis (peak flow verocity:3.73 m/s) was also pointed out. The redo double valve replacement was performed. Intraoperative findings showed discrete type subaortic stenosis due to extensive pannus formation, but that the previously implanted prosthetic valves were intact. The blood flow biased by the interference of the subaortic stenosis might have obstructed closure of the mitral prosthetic valve and caused mitral regurgitation. Postoperatively, hemolysis and mitral regurgitation were diminished, and aortic stenosis was improved.

Diagnosis and surgical management of subaortic stenosis and mitral valve systolic anterior motion.

The case is reported of a patient with a previously undiagnosed cause of severe congestive heart failure (CHF) caused by the presence of a discrete subaortic stenosis (SAS) from a subvalvular membrane (SVM). The clinical decision making was complicated by the concurrent presence of systolic anterior motion (SAM) of the mitral valve leaflet. Due to the limitations and eventual failure of physiologically opposing medical management strategies, the patient eventually required an open-heart surgical approach and underwent intraoperative SVM resection. A persistent intraoperative left ventricular outflow tract (LVOT) gradient of 50 mmHg due to SAM prompted mitral valve replacement, which resulted in a complete resolution of the LVOT gradient and symptoms. In this extremely rare scenario of SAS and SAM, when SVM resection is thought to be inadequate to relieve LVOT obstruction due to the concurrent presence of SAM, mitral valve replacement represents a reasonable therapeutic approach.